Ch. 18 kidney and urinary tract disorders.

Proteinuria. Persistent proteinuria is significant and should be quantified by measuring the urine protein/creatinine ratio in an early morning sample (protein should not exceed 20mg/mmol of creatinine). A common cause is orthostatic (postural) proteinuria, where proteinuria is only found when the child is upright. It can be diagnosed by measuring the urine protein in a series of morning urine specimens. Prognosis is excellent. Nephrotic syndrome. Heavy proteinuria results in a low plasma albumin, and oedema. Clinical signs are: - Periorbital oedema; - scrotal or vulval , leg and ankle oedema; - Ascites; - Breathlessness due to pleural effusions. In 85-90% of children the proteinuria resolves with corticosteroid therapy. These children do not progress to renal failure. The most widely used protocol is to initially give oral corticosteroids. The median time for the urine to become free of protein is 11 days. Children who do not respond to 4-8 weeks of corticoid therapy or have atypical features may have a more complex diagnosis and require a renal biopsy. The child is susceptible to several serious complications: - Hypovolemia: during the initial phase of oedema they become volume depleted. The child characteristically complains of abdominal pain and may feel faint. There is peripheral vasoconstriction and urinary sodium retention. It requires urgent treatment with intravenous albumin as the child is at risk of vascular thrombosis and shock. - Thrombosis: a hypercoagulable state, due to urinary losses of antithrombin, which may be exacerbated by steroid therapy. - Infection: children in relapse are at risk of infection with capsulated bacteria, especially pneumococcus. - Hypercholesterolaemia: this correlates inversely with the serum albumin. Congenital nephrotic syndrom. Presents in the first 3 moths of life. It is rare. It is associated with a high mortality, usually due to complications of hypoalbuminaemia rather than renal failure. Heamaturia. Urine is red in colour or tests positive for haemoglobin on urine sticks should be examined under the microscope to confirm haematuria. Glomerular haematuria is suggested by brown urine, the presence of deformed red cells and casts, and is often accompanied by proteinuria. Lower urinary tract haematuria is usually red, occurs at the beginning or end of the urinary stream, is not accompanied by proteinuria and is unusual in children. Urinary tract infection is the most common cause. A renal biopsy may be indicated if: - Significant persistent proteinuria; - Recurrent macroscopic heamaturia; - Renal function is abnormal. Acute nephritis. Restricts glomerular blood flow and therefore filtration is decreased. This leads to: - Decreased urine output and volume overload; - Hypertension; - Oedema; - Haematuria and proteinuria. Post-streptococcal and post-infectious nephritis: follows a streptococcal sore throat or skin infection and is diagnosed by evidence of a recent streptococcal infection and low complement C3 levels that return to normal after 3-4 weeks. Henoch-Schonlein purpura: combination of the following features,

- Characteristic skin rash; - Arthralgia; - Periarticular oedema; - Abdominal pain; - Glomerulonephritis. It occurs between the ages of 3-10 years, peaks during winter. The IgA and IgG interact to produce complexes that activate complement and are deposited in affected organs. The affected children often have a fever. The rash is the most obvious feature. It is symmetrically distributed over the buttocks, the extensor surfaces of the arms and legs, and the ankles. Joint paint occurs in 2/3 of patients. There is periarticular oedema. Colicky abdominal pain occurs in many children and if severe can be treated with corticosteroids. Renal involvement is common, but it is rarely the first symptom. Over 80% have microscopic or macroscopic heamaturia or mild proteinuria. Risk factors for progressive renal disease are heavy proteinuria, oedema, hypertension and deteriorating renal function. Vasculatitis: commonest vasculitis to involve the kidney is Henoch-schonlein purpura. However, renal involvement may occur in rare vasculitides such as polyarteritis nodosa, microscopic polyarteritis and wegener granulomatosis. Characteristic symptoms are fever, malaise, weight loss, skin rash and arthropathy. Systemic lupus erythematosus (SLE): presents mainly in adolescent girls and young women. It is characterised by the presence of multiple autoantibodies, including antibodies to double stranded DNA. The C3 and C4 components of complement may be low. Heamaturia and proteinuria are indications for renal biopsy. Hypertension. Blood pressure needs to be measured with a cuff over 2/3 the length of the upper arm. Symptomatic hypertension in children is usually secondary to renal, cardiac or endocrine causes. Presentation includes vomiting, headaches, facial palsy, hypertensive retinopathy, convulsions or proteinuria. Early detection is important. Renal: - Renal parenchymal disease; - Renovascular, e.g. renal artery stenosis; - Polycystic kidney disease; Coarctation of the aorta. Catecholamine excess: - Phaeochromocytoma; - Neuroblastoma; Endocrine: - Congenital adrenal hyperplasia; - Cushing syndrome Essential hypertension. Renal masses. Bilaterally enlarged kidneys in early life are most frequently due to autosomal recessive polycystic kidney disease, which is associated with hypertension, hepatic fibrosis and progression to chronic renal failure. This form must be distinguished from the autosomal dominant adult-type polycystic kidney disease. Renal calculi. Renal stones are uncommon in childhood, predisposing causes must be sought: - Urinary tract infection; - Structural anomalies of the urinary tract; - Metabolic abnormalities. Commonest are phosphate stones associated with infection. Calcium-containing stones occur in idiopathic hypercalciuria. Stones that are not passed spontaneously should be removed, by either lithortipsy or

surgery, and any predisposing structural anomaly repaired. Renal tubular disorders. General proximal tubular dysfunction (fanconi syndrome). Proximal tubule cells are among the most metabolically active in the body, especially vulnerable to cellular damage. Fanconi syndrome should be considred in a child present with: - Polydipsia and polyuria; - Salt depletion and dehydration; - Hyperchloraemic metabolic acidosis; - Rickets; - Failure to thrive/poor growth. Acute kidney injury. There is sudden, potentially reversible, reduction in renal function. Oliguria (<0,5 ml/kg per hour) is usually present. Prerenal: the commonest cause in children; Renal: there is salt and water retention; Postrenal: from urinary obstruction. The circulation and fluid balance should be monitored. Prerenal failure is suggested by hypovolaemia. The fractional excretion of sodium is very low as the body tries to retain fluid. Renail failure: if there is circulatory overload, restriction of fluid intake and challenge with diuretic may increase urine output. A high-calorie, normal protein feed will decrease catabolism, uraemia and hyperkalaemia. The two commonest renal causes are haemolytic uraemic syndrome and acute tubular necrosis. Postrenal failure: requires assessment of the site of obstruction and relief by nephrostomy or bladder catheterisation. Dialysis is indicated when there is: - Failure of conservative management; - Hyperkalaemia; - Severe hypo- or hypernatraemia; - Pulmonary oedema or hypertension; - Severe acidosis; - MOF. Haemolytic uraemic syndrome (HUS). Is a triad of acute renal failure, microangiopathic haemolytic anaemia and thrombocytopenia. Typical HUS is secondary to gastrointestinal infection with verocytotoxin producing E.coli . The toxin from these organisms enters the gastrointestinal mucosa and preferentially localises to the endothelial cells of the kidney where it causes intravascular thrombogenesis. Platelets are consumed in this process and microangiopathic haemolytic anaemia results from damage to red blood cells are they circulate through the microcirculation. Chronic kidney disease. GFR <15 ml/min per 1.73 m^2, is much less common in children. Clinical features are: - Anorexia and lethargy; - Polydipsia and polyuria; - failure to thrive/ growth failure; - Bony deformities.

Many children have had their renal disease detected before birth by antenatal ultrasound. The aims are to prevent the symptoms and metabolic abnormalities of chronic renal failure, to allow normal growth and development.